title: Differences in disease expression between primary ciliary dyskinesia and cystic fibrosis with and without pancreatic insufficiency.
abstract: BACKGROUND
  Impaired mucociliary clearance causes pulmonary disease in primary ciliary dyskinesia (PCD) and contributes to cystic fibrosis (CF) lung disease. Although the sinopulmonary disease is similar, morbidity and mortality are different. Both patients with PCD and patients with CF with pancreatic sufficiency (CF-PS) show no nutrient malabsorption and are diagnosed at a later age compared with patients with CF with pancreatic insufficiency (CF-PI).
  
  
  METHODS
  Clinical status, microbiology, FEV1, and high-resolution CT (HRCT) scans presented as total Brody score (CT-TBS) were compared for patients with PCD, CF-PI, and CF-PS, all treated at the same medical center, by the same team, and by a similar routine follow-up.
  
  
  RESULTS
  One hundred sixty-four patients, 34 with PCD, 88 with CF-PI, and 42 with CF-PS were enrolled. PCD was diagnosed at a similar age as CF-PS but significantly later than CF-PI. Mean FEV1 % predicted was similar for the three groups. The rate of FEV1 change with age in PCD was similar to CF-PS but significantly lower than in CF-PI. Severity of structural lung disease (CT-TBS) was similar for PCD and CF-PS and significantly higher in CF-PI. No correlation between TBS or Pseudomonas aeruginosa infection and FEV1 in PCD was seen, whereas a negative correlation with FEV1 was observed for both CF groups.
  
  
  CONCLUSIONS
  Although in our study PCD was similar to CF-PS, the lack of correlation between FEV1 and age, CT-TBS, and P aeruginosa infection in PCD suggests that impaired mucociliary clearance is not the only cause for inducing pulmonary damage in these diseases. Furthermore, a comparison of disease characteristics for PCD and CF should distinguish between CF-PI and CF-PS as different entities.
authors.affiliation: Department of Pediatrics, Hadassah Hebrew-University Medical Center, Mount Scopus, Jerusalem, Israel; Cystic Fibrosis and Primary Ciliary Dyskinesia Center, Hadassah Hebrew-University Medical Center, Mount Scopus, Jerusalem, Israel.
authors.forename: Malena
authors.initials: M
authors.lastname: Cohen-Cymberknoh
authors.affiliation: Department of Radiology, Hadassah Hebrew-University Medical Center, Mount Scopus, Jerusalem, Israel.
authors.forename: Natalia
authors.initials: N
authors.lastname: Simanovsky
authors.affiliation: Department of Radiology, Hadassah Hebrew-University Medical Center, Mount Scopus, Jerusalem, Israel.
authors.forename: Nurith
authors.initials: N
authors.lastname: Hiller
authors.affiliation: Department of Pediatrics, Hadassah Hebrew-University Medical Center, Mount Scopus, Jerusalem, Israel.
authors.forename: Alex Gileles
authors.initials: AG
authors.lastname: Hillel
authors.affiliation: Department of Pediatrics, Hadassah Hebrew-University Medical Center, Mount Scopus, Jerusalem, Israel; Cystic Fibrosis and Primary Ciliary Dyskinesia Center, Hadassah Hebrew-University Medical Center, Mount Scopus, Jerusalem, Israel.
authors.forename: David
authors.initials: D
authors.lastname: Shoseyov
authors.affiliation: Department of Pediatrics, Hadassah Hebrew-University Medical Center, Mount Scopus, Jerusalem, Israel; Cystic Fibrosis and Primary Ciliary Dyskinesia Center, Hadassah Hebrew-University Medical Center, Mount Scopus, Jerusalem, Israel. Electronic address: kerem@hadassah.org.il.
authors.forename: Eitan
authors.initials: E
authors.lastname: Kerem
mesh_terms: D000293:Adolescent; D003430:Cross-Sectional Studies; D003550:Cystic Fibrosis / Q000150:complications* / Q000175:diagnosis*; D010188:Exocrine Pancreatic Insufficiency / Q000150:complications* / Q000175:diagnosis*; D005260:Female; D006801:Humans; D007619:Kartagener Syndrome / Q000150:complications* / Q000175:diagnosis*; D008297:Male